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Splanchnic vein thrombosis: clinical presentation, risk factors and treatment

Valerio De Stefano1, Ida Martinelli

  • 1Institute of Hematology, Catholic University, Largo Gemelli 8, 00168 Rome, Italy. valerio.destefano@rm.unicatt.it

Internal and Emergency Medicine
|June 10, 2010
PubMed

Insights

Splanchnic vein thrombosis, including Budd-Chiari syndrome (BCS) and portal vein obstruction (EHPVO), often involves multiple risk factors like myeloproliferative neoplasms and inherited thrombophilia. Treatment involves anticoagulation, with advanced procedures for severe cases.

Area of Science:

  • Vascular Medicine
  • Hematology
  • Thrombosis Research

Background:

  • Splanchnic vein thrombosis (SVT) encompasses Budd-Chiari syndrome (BCS), extrahepatic portal vein obstruction (EHPVO), and mesenteric vein thrombosis.
  • While rare, SVT incidence may be underestimated, with non-invasive imaging detecting chronic or asymptomatic cases.
  • Risk factors for SVT are diverse, including local factors (common in portal vein thrombosis) and systemic causes.

Purpose of the Study:

  • To review the epidemiology, risk factors, and management of splanchnic vein thrombosis.
  • To highlight the significant role of chronic myeloproliferative neoplasms (MPN) and inherited thrombophilia in SVT.
  • To outline current treatment strategies, including anticoagulation and interventional procedures.

Main Methods:

  • Review of existing literature on splanchnic vein thrombosis, Budd-Chiari syndrome, and portal vein obstruction.
  • Analysis of epidemiological data and risk factor prevalence, including MPN and thrombophilia mutations.
  • Summary of current therapeutic approaches, including anticoagulation, thrombolysis, and shunt placement.

Main Results:

  • Chronic myeloproliferative neoplasms (MPN) are a leading cause, found in a significant proportion of BCS and EHPVO patients.
  • The JAK2 V617F mutation is prevalent in MPN-associated SVT.
  • Inherited thrombophilias, such as Factor V Leiden and prothrombin G20210A mutations, are present in at least one-third of patients.
  • Multiple risk factors are common, particularly in portal vein thrombosis.

Conclusions:

  • Splanchnic vein thrombosis requires comprehensive evaluation for both local and systemic risk factors, especially MPN and inherited thrombophilia.
  • Acute management involves anticoagulation, potentially combined with thrombolysis or shunting for severe cases.
  • Long-term anticoagulation is crucial for BCS and selected EHPVO patients to prevent recurrence.

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